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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 5  |  Page : 1017-1018
Diarrhea-related hemolytic uremic syndrome: Unmasking antifactor H antibodies

Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication6-Sep-2011


The cases of hemolytic uremic syndrome (HUS) preceeded by diarrhea are commonly labeled as D+ HUS. However, with severe renal failure or with delayed recovery, such cases should be evaluated for rarer associations like Factor H (FH), I and CD 46 deficiency or mutations. We report such a presentation of a young boy who initially came with diarrhea and had features of HUS with delayed renal recovery. He later turned out to have anti-FH antibody-related HUS.

How to cite this article:
Gupta A, Khaira A, Rathi OP, Mahajan S, Bhowmik D, Agarwal SK, Tiwari SC. Diarrhea-related hemolytic uremic syndrome: Unmasking antifactor H antibodies. Saudi J Kidney Dis Transpl 2011;22:1017-8

How to cite this URL:
Gupta A, Khaira A, Rathi OP, Mahajan S, Bhowmik D, Agarwal SK, Tiwari SC. Diarrhea-related hemolytic uremic syndrome: Unmasking antifactor H antibodies. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2022 May 16];22:1017-8. Available from: https://www.sjkdt.org/text.asp?2011/22/5/1017/84525

   Introduction Top

Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It presents as diarrheal and non-diarrheal (atypical) forms. The usual course of diarrheal HUS is renal recovery, while in patients with non-diarrheal HUS, recovery of renal function is much less common. Among such cases, absolute or functional deficiency of Factor H (FH) forms an important cause. We herewith report a case of HUS who had anti-FH antibody-related HUS.

   Case Report Top

A 14-year-old boy was admitted with fever and diarrhea of three days duration. He was treated with oral fluids and ciprofloxacin at a local health center. On the third day of his illness, he developed progressively decreasing urine output and was referred to our hospital. On admission, he was oliguric, hypertensive, euhydrated and pale with systemic examination being essentially normal. Investigations revealed hemoglobin of 7.9 g/dL, leukocyte count of 10,300/mm [3] , platelet count of 67,000/mm [3] and corrected reticulocyte count of 2.5%; blood film showed schistocytes with normal coagulation profile. The blood urea was 167 mg/dL, serum creatinine was 7.7 mg/dL, serum bilirubin was 2.3 mg/dL (predominantly of the unconjugated type), alanine aminotransferase was 67 U/L, aspartate aminotransferase was 204 U/L and lactate dehydrogenase was 934 U/L. Prothrombin time, activated partial thromboplas tin time and fibrin degradation products were normal. Urine routine showed protein +, white blood cells 3-5 and red blood cells 0-1/hpf. Stool culture was negative. Ultrasound revealed normal-sized kidneys. The anti-nuclear and anti-neutrophil cytoplasmic antibodies and C4 levels were normal. C3 antigen was 542 (normal 660-1250 mg/L 2 ). An arteriovenous shunt was made and hemodialysis was started. He continued to be oligo-anuric and dialysis dependent, and a percutaneous renal biopsy performed ten days later showed features of thrombotic microangiopathy. FH (87%) and Factor I (95%) determined by enzyme-linked immunosorbent assay and CD 46 levels, determined by mean of fluorescence intensity, were normal. Anti-FH auto-antibodies were present in high titers (14,000 arbitrary units), with reference plasma titers being 1000 AU/mL. He was started on plasma exchange with fresh frozen plasma as replacement fluid. A total of ten sessions of plasma exchange were given over the next two weeks. The patient's hematological parameters resolved, but he remained anuric and dialysis dependent.

   Discussion Top

Our patient presented with atypical HUS associated with diarrhea. Such an uncommon presentation has only been reported once. [1] The delay in recovery prompted us to investigate the possibility of rarer associations. FH mutations and anti-FH auto-antibodies are known to occur in D-HUS. Although mutations in the FH gene are described more often, our patient had anti-FH antibodies leading to an acquired functional FH deficiency. These auto-antibodies may influence binding of FH to the C3b,Bb convertase, thereby compromising FH activity in vivo. [2] As reported among hereditary FH-deficient individuals, our case presented with low C3 levels, suggestive of mild alternative pathway-mediated complement activation.

Our case is unique as the diagnosis of D+ HUS may have lead to disastrous renal transplantation. The risk of graft loss approaches 80% in such cases. [3] FH is produced by the liver and combined liver-kidney transplantation could be one option. However, plasma exchange could prove useful in removal of the anti-FH antibodies, although in our case we could not achieve renal recovery. Rituximab was one option that could have been tried, but was not given due to financial constraints. [4]

To conclude, cases labeled as D+ HUS who have severe renal failure or where renal functions fail to recover should be evaluated further for the possibility of atypical HUS. Identification of the specific genetic defect in patients with atypical HUS should improve diagnostic precision and help to predict clinical outcomes. This would help to decide the further plan of renal replacement therapy in such cases.

   Acknowledgments Top

The authors would like to thank Arvind Bagga and Siddhartha Sethi of the Department of Pediatric Nephrology, All India Institute of Medical Sciences and Marie-Agnes Dragon-Durey of INSERM U430, Institut des Cordeliers, Paris, France.

   References Top

1.Edey MM, Mead PA, Saunders RE, et al. Association of a factor H mutation with hemolytic uremic syndrome following a diarrhoeal illness. Am J Kidney Dis 2008;51:487-90.  Back to cited text no. 1
2.Dragon-Durey MA, Loirat C, Cloarec S, et al. Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 2005;16:555-63.  Back to cited text no. 2
3.Zimmerhackl LB, Scheiring J, Prüfer F, Taylor CM, Loirat C. Renal transplantation in HUS patients with disorders of complement regulation. Pediatr Nephrol 2007;22:10-6.  Back to cited text no. 3
4.Kwon T, Dragon-Durey MA, Macher MA, et al. Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome. Nephrol Dial Transplant 2008;23:2088-90.  Back to cited text no. 4

Correspondence Address:
Ankur Gupta
Department of Nephrology, All India Institute of Medical Sciences, New Delhi 110029
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Source of Support: None, Conflict of Interest: None

PMID: 21912037

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